89A Case of Severe Guillain-Barré Syndrome ...

Correspondence & reprint requests to: Dr. Daad H. Akbar, P. O. Box 18298, Jeddah 21415, Saudi Arabia.e-mail: daadakb@yahoo.com

Accepted for publication: 12 May, 2001. Received: 05 February, 2001

89

Severe Guillain-Barré Syndrome which Responded to Repeated Courses of Intravenous Immunoglobulin:

A Case Report

DAAD H, AKBAR, FRCP, Arab Board, Saudi Board

Department of Medicine, Faculty of Medicine & Allied Sciences, King Abdulaziz University, Jeddah, Saudi Arabia

ABSTRACT. Guillain-Barré Syndrome is an idiopathic acute inflammatory de-mylinating polyneuropathy with good prognosis. We described a case of a 56-years old male patient was presented with flaccid quadriplegia and respiratoryfailure due to Guillain-Barré Syndrome which was successfully treated with re-peated courses of intravenous immunoglobulin. Further studies are needed onthis modality of treatment especially in severe form of Guillain-Barré Syn-drome.

Keywords. Severe Guillain-Barré Syndrome, Respiratory failure, Intravenousimmunoglobulin

Introduction

Guillain-Barré Syndrome (GBS) is an idiopathic acute inflammatory demyelinatingpolyneuropathy characterized by progressive muscle weakness and a relexia with spon-taneous remission being the rule[1]. It is a leading cause of acute paralysis. It occursworld-wide in patients of all ages and both sexes[2, 3]. It has an annual incidence of 1.2cases per 100,000 population[4] and a mortality of 3-8%[5-7]. About one-third of pa-tients require intensive care unit admission for assisted ventilation and management ofautonomic cardiovascular instability[5]. Several studies have shown the efficacy of in-travenous immunoglobulin (IVIg) in the treatment of GBS [8-10].

J KAU: Med. Sci., Vol. 10, pp. 89-92 (1422 A.H. / 2002 A.D.)

D. Akbar90

We described a case of a 56-years old male who presented himself with severe GBS(flaccid quadriplegia ad respiratory failure) that had been successfully treated with re-peated courses of IVIg.

Case Report

A fifty-six (56) years old Saudi male patient was admitted to our hospital with a his-tory of acute respiratory distress. His problem started 2 weeks before admission with ahistory of upper respiratory tract infection followed 10 days later by weakness in bothlower limbs that progressively ascended to the upper limbs followed by the develop-ment of severe difficulty in breathing within one week of the start of his illness. Hissystemic review didn’t reveal significant abnormalities. He has been a diabetic for 2years and his blood glucose is well controlled with oral hypoglycemic agents. He had ahistory of abdominal surgery, 10-years ago for removal of colonic mass. He is a clerk,married with 4 children, and is a non-smoker. His family and allergic history were un-remarkable. On examination, the patient was in severe respiratory distress, tachycardia(pulse 140/min.), blood pressure 140/90 mmHg, and his neurological examination re-vealed flaccid quadriplegia grade II. Cranial nerves’ examination was intact and fundiwere normal. Heart and chest examinations were clear. Abdominal examination re-vealed non-tender hepatomegaly with liver span of 16 cm. The patient was intubatedand his initial investigations showed normal complete blood count, serum urea andcreatinine, liver function tests, chest X-ray, and controlled blood glucose. His electro-cardiography showed persistent sinus tachycardia. Lumber puncture was performedand it showed raised cerebrospinal fluid protein with normal cells. Electro-physiological nerve study’s findings were consistent with the diagnosis of GBS withdemyelinating feature. Cytomegalovirus and Epstein-Bar virus serology tests werenegative. Abdominal ultrasound showed adenocarcinomata secondaries. The patientwas treated with IVIg course (0.4 gm/kg/day) for 5 days, and beta-blocker (metoprolol)was added to control his sinus tacchycardia in addition to other supportive measures.The patient didn’t show significant improvement neither in motor nor respiratory func-tion. To control the underlying immunological abnormalities another course of IVIg(0.4 gm/kg/day) for 5 days was considered after 2 weeks. The patient showed re-markable improvement in his respiratory function within a few days and we were ableto wean him from the ventilator. Also, he showed progressive improvement in motorfunction and was able to walk around without support. An oncologist’s opinion wastaken regarding the patient’s liver metastasis. After 3 months of hospital’s admission,the patient was discharged home in good general condition, breathing spontaneouslyand walking unassisted. He is on regular follow-up for 2 years now after his illness.He is doing fine without any motor or respiratory sequelae and without any relapses.

Discussion

GBS is an autoimmune disease of peripheral nerves. Suffers may require intensivecare and may be permanently disabled[11]. GBS has a more benign course in children[12] and severe forms of adult GBS had been reported in patients with mean age of 48years [6]. Increasing age and severe rapidly progressive disease had been found to besignificantly associated with poor outcome[4, 7, 13]. Antecedent infection whether res-piratory or gastrointestinal has been reported in approximately two-third of patients[1,7]. Our patient was in the mid-fifties with a history of proceeding respiratory tract in-fection and rapidly progressive disease with respiratory failure within one week of theonset of his illness. Autonomic dysfunction is a well organized feature of GBS and is asignificant source of mortality[14]. It has been described in 11% of patients with severe

91A Case of Severe Guillain-Barré Syndrome ...

GBS[6]. Our patient had persistent sinus tacchycardia due to autonomic dysfunction.The course of GBS is believed to be immune mediated, a variety of clinical and experi-mental data have implicated both humoral factor and cell mediated immune phenomenawhich damage myelin and/or myelin-producing Schwann cells[15]. The main mo-dalities of therapy for GBS is to control the immune abnormality by either plasma pher-esis (PE) or IVIg. Intravenous immunoglobulin has been advocated for therapy of GBSas it is effective, safe, and easy to use[8, 10, 16]. Combination of PE and IVIg in severedisease didn’t confer a significant advantage over single therapy, but it increases costand risk [9]. Our patient showed dramatic response to repeated course IVIg without se-quelae or relapses. Farcas et al.[17] had described a remarkable recovery after the sec-ond course of IVIg in a case of severe GBS. This raises the possibility that repeatedcourses of IVIg might be helpful in severe GBS. Further studies are needed on this mo-dality of therapy especially in severe forms of GBS.

References

[1] Hahn AF. Guillain-Barré syndrome. Lancet 1998; 352: 635-641.[2] Hughes ARC. Guillain-Barré Syndrome. London: Springer-Verlag, 1990.[3] Ropper JH, Wijdicks EFM, Truax BT. Guillain-Barré Syndrome. Volume 34 of Contemporary

Neurology Series. Philadelphia, PA USA: FA Davis, 1991.[4] Rees JH, Thompson RD, Smeeton NC, Hughes RA. Epidemiological study of Guillain-Barré syn-

drome in southeast England. J Neurol Neurosurg Psychiatry 1998; 64(1): 74-77.[5] Hahn AF. Management of Guillain-Barré Syndrome (GBS). Baillieres Clin Neurol 1996; 5(3): 627-

644.[6] Ng KK, Howard RS, Fish DR, Hirsch NP, Wiles CM, Murray NM, Miller DH. Management and

outcome of severe Guillain-Barré Syndrome. QJM 1995; 88(4): 243-250.[7] (No authors listed). A prospective study on the inidence and prognosis of Guillain-Barre syndrome in

Emilia-Romagna region, Italy (1992-1993). Emilia-Romagna Study Group on Clinical and Epidemi-ological Problems in Neurology. Neurology 1997; 48(1): 214-221.

[8] Sater RA, Rostami A. Treatment of Guillain-Barré syndrome with intravenous immunoglobulin.Neurology 1998; 51 (6 Supp. 5): S9-15.

[9] (No authors listed). Randomised trial of plasma exchange, intravenous immunoglobulin, and com-bined treatments in Guillain-Barre syndrome. Plasma Exchange/Sandoglobulin Guillain-Barré Syn-drome Trial Group. Lancet 1997; 349: 225-230.

[10] Fasanaro AM, Pizza V, Rossi V. Immunoglobulins i.v.: a new approach to the treatment of Guillain-Barré syndrome. Minerva Med 1996; 87(1-2): 17-20.

[11] Hadden RD, Hughes RA. Guillain-Barré syndrome: recent advances. Hosp Med 1998; 59(1): 55-60.

[12] Gurses N, Uysal S, Cetinkaya F, Islek I, Kalayci A. Intravenous immunoglobulin treatment in chil-dren with Guillain-Barré syndrome. Scan J Infect Dis 1995; 27: 241-3.

[13] Ropper AH. The Guillain-Barré syndrome. N Engl J Med 1992; 326(17): 1130-1136.[14] Hund EF, Borel CO, Cornblath DR, Hanley DF, McKhann GM. Intensive management and treat-

ment of severe Guillain-Barré syndrome. Crit Care Med 1993; 21(3): 433-446.[15] Saida T, Saida K, Dorfman SH, Silberberg DH, Sumner AJ, Manning MC, Lisak RP. Experi-

mental allergic neuritis induced by sensitization with galactocelebroside. Science 1979; 204: 1103-1106.

[16] van der Meche FG. The Guillain-Barré syndrome: diagnosis, treatment and prognosis. Acta NeurolBelg 1994; 94(2): 124-127.

[17] Farcas P, Avnun L, Frisher S, Herishanu YO, Wirgiun I. Efficacy of repeated intravenous im-munoglobulin in severe unresponsive Guillain-Barré syndrome. Lancet 1997; 350(9093): 1747.

D. Akbar92

��dJ��  U�d' X�U���« w��« Í�U� 5�KO'« ÷d* �dD� W�U��lML*« 5�Ëd��« s�

d��√ s�� b�� e�eF�«b�� pK*« WF�U� , WO�D�« ÂuKF�«Ë VD�« WOK� , VD�« r��

W��uF��« WO�dF�« WJKL*« − �b����

t�U?��√ Æ»U?B�ú� �U?� »UN?��« u� Í�U� 5KO'« ÷d?� ÆhK�?��*«W�U?� n6Ë - bI� ÆW?O�U� t?O� ¡U?HA�« W?��� ÊuJ� U?� ��U�Ë W?�ËdF?� dO?��dD)« t��u?B� Í�U� 5KO?'« ÷d0 VO6√ WM� µ∂ d?L?F�« s� mK�� q��t?�ö� b?F� Á¡U?H� - b?�Ë fHM��« w� �u?B?�Ë ·«d�ú� w�U�� qK� l�W?OK�?I?�?�?�  U?�«�� qL� V?�� Æ lML*« 5�Ëd?��« s� ��dJ�?�  U?�d?��Í�U� 5KO??'«  ôU?� w?� U?N?�??O�U??F?�  U??��ù Ãö?F�« s� W??I�dD�« Ác??N�

Æ��dD)«